Abstract: Nine cases of severe hydroa vacciniforme are reported.All the cases had erythema, papules, bullae, erosions or ulcers on exposed sites, with recurrences and gradually resulted in deformation.Five patients suffered from hand deformation manifesting rigidity, flexor tetanus or malposition of the first, second and third interphalangeal joints of hands.Two of the above mentioned patients manifested partial bone absorption of fingers.Five patients showed partial defect of auricle.Two patients showed saddle nose, and partial absorption of nasal cartilage.One patient displayed cica trical contracture of lower lip, with incisor extrusion.Four patients revealed opacity of cornea.Through laboratory examinations, porphyrinopathies were excluded.Phototest showed increased sensitivity to UVA.Based on the clinical features and laboratory examinations,these patients were diagnosed as severe hydroa vacciniforme.It is believed that summer prurigo, hydroa aestivale, hydroa vacciniforme and severe hydroa vacciniforme all belong to a disease spectrum induced by UV light.

Key words: Hydroa vacciniforme